Diffuse panbronchiolitis

A rare chronic inflammatory OBSTRUCTIVE PULMONARY DISEASE that occurs more frequently in persons of East Asian descent. Onset is in adulthood or middle age and is characterized by INFLAMMATION of the BRONCHIOLES, chronic cough, exertional DYSPNEA, and SPUTUM production. Most patients also have chronic paranasal SINUSITIS, Haemophilus influenzae and PSEUDOMONAS AERUGINOSA infections. If untreated, the disorder progresses to BRONCHIECTASIS and RESPIRATORY FAILURE. It has been mapped to a region of chromosome 6. OMIM: 604809

Also Known As:

Networked: 330 relevant articles (64 outcomes, 27 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cystic Fibrosis (Mucoviscidosis)
2.	Bronchiectasis
3.	Asthma (Bronchial Asthma)
4.	Sinusitis
5.	Respiratory Tract Diseases (Respiratory Tract Disease)

Experts

1.	Kohno, Shigeru: 13 articles (01/2012 - 05/2002)
2.	Mukae, Hiroshi: 12 articles (12/2022 - 05/2002)
3.	Kudoh, Shoji: 10 articles (01/2019 - 01/2002)
4.	Yanagihara, Katsunori: 9 articles (01/2012 - 05/2002)
5.	Tomono, Kazunori: 9 articles (01/2007 - 05/2002)
6.	Azuma, Arata: 7 articles (10/2016 - 12/2002)
7.	Kohno, S: 7 articles (07/2003 - 01/2000)
8.	Kadota, Jun-Ichi: 6 articles (02/2019 - 05/2002)
9.	Hirakata, Yoichi: 6 articles (01/2007 - 05/2002)
10.	Miyazaki, Yoshitsugu: 6 articles (01/2007 - 05/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Diffuse panbronchiolitis:

1.	MacrolidesIBA 01/01/2007 - "Chronic, low-dose macrolides have dramatically improved survival in patients with diffuse panbronchiolitis (DPB). " 04/01/2004 - "Macrolides have been shown to be effective in treating diffuse panbronchiolitis (DPB), although the precise modes of action remain unclear. " 02/01/2004 - "This led to studies of the macrolides in other chronic airway diseases, such as diffuse panbronchiolitis (DPB), a disease occurring primarily in East Asia. " 01/01/2016 - "Benefits of macrolide usage and bacteriological profile in patients with diffuse panbronchiolitis." 01/25/2015 - "Macrolides for diffuse panbronchiolitis."
2.	Erythromycin (Erycette)FDA LinkGeneric 03/01/1998 - "The prognosis for diffuse panbronchiolitis has dramatically improved over the past 10 years with the use of low-dose long-term erythromycin treatment. " 03/01/2001 - "The mechanisms underlying the therapeutic efficacy of erythromycin (EM) in diffuse panbronchiolitis (DPB) was investigated. " 03/01/1996 - "Diffuse panbronchiolitis has been reported to occur in the United States and Europe, and the anti-inflammatory action of erythromycin appears to be effective in management. " 02/01/1994 - "Recently, "low-dose and long-term" erythromycin (EM) has been reported to be effective in treatment of diffuse panbronchiolitis (DPB), but its mechanism is still obscure. " 11/05/2004 - "Diffuse panbronchiolitis (DPB) is a chronic progressive disease of the respiratory bronchioles, and has been improved by low-dose, long-term erythromycin (EMC) treatment. "
3.	Anti-Bacterial Agents (Antibiotics)IBA 11/27/2001 - "In addition to their antibacterial action, this class of antibiotics exhibits anti-inflammatory activity in a variety of airway diseases such as asthma and diffuse panbronchiolitis that is separate and distinct from a direct antibacterial action. " 08/01/1983 - "[Antibiotic therapy against respiratory infections in diffuse panbronchiolitis]." 10/01/2003 - "Long-term treatment of macrolide antibiotics is considered an effective treatment for diffuse panbronchiolitis (DPB). " 06/01/2007 - "Macrolide antibiotics have demonstrated efficacy in diffuse panbronchiolitis, a disease that shares many similarities to CF, and this has generated interest in the use of these agents in patients who have CF. " 04/01/2020 - "The first clinical indication of non-antibiotic benefits of macrolides was in the Far East, in adults with diffuse panbronchiolitis. "
4.	Azithromycin (Zithromax)FDA LinkGeneric 01/01/2012 - "Evidence of improved small airways function after azithromycin treatment in diffuse panbronchiolitis." 06/01/1995 - "[Study on azithromycin in treatment of diffuse panbronchiolitis]." 02/01/2019 - "Azithromycin treats diffuse panbronchiolitis by targeting T cells via inhibition of mTOR pathway." 10/01/2013 - "The effects of azithromycin on patients with diffuse panbronchiolitis: a retrospective study of 29 cases." 01/01/2012 - "A 67-year-old never-smoker was diagnosed with diffuse panbronchiolitis (DPB) and was started on 250 mg azithromycin twice weekly. "
5.	RoxithromycinIBA 05/01/1992 - "[A case of diffuse panbronchiolitis relieved rapidly by the treatment of roxithromycin]." 01/01/1994 - "We demonstrated the efficacy of "long term" roxithromycin (RXM) treatment in 15 patients with chronic lower respiratory tract disease (11 with diffuse panbronchiolitis and 4 with sinobronchial syndrome). " 01/01/1995 - "To evaluate the effects of roxithromycin in patients with chronic lower respiratory tract disease including diffuse panbronchiolitis, we studied lymphocyte function-associated antigen-1 (LFA-1) and Mac-1, neutrophil adhesion molecules, using peripheral neutrophils from healthy volunteers and from patients with chronic lower respiratory tract disease. " 06/01/1996 - "In the present study, we demonstrated the efficacy of low-dose, long-term therapy with erythromycin and an erythromycin-related compound, roxithromycin, in patients with chronic lower respiratory tract disease including diffuse panbronchiolitis (DPB), and investigated the anti-inflammatory action of such drugs. " 01/01/2011 - "Patients with diffuse panbronchiolitis (DPB) are routinely treated with erythromycin, clarithromycin, and roxithromycin. "
6.	Clarithromycin (Biaxin)FDA LinkGeneric 01/01/1989 - "[Long-term administration study on TE-031 (A-56268) in the treatment of diffuse panbronchiolitis]." 07/01/2003 - "Long-term efficacy and safety of clarithromycin treatment in patients with diffuse panbronchiolitis." 02/01/2019 - "The development of diffuse panbronchiolitis during the treatment with long-term, low-dose clarithromycin for chronic sinusitis." 01/01/1997 - "In a newly established murine model of chronic Pseudomonas aeruginosa respiratory infection mimicking diffuse panbronchiolitis (DPB), we investigated the effect of oral administration of clarithromycin on lymphocyte accumulation in the lung. " 09/01/2016 - "Historically, erythromycin and clarithromycin were found to stabilize pulmonary deterioration in diffuse panbronchiolitis. "
7.	AntigensIBA 06/01/1990 - "These increases might be explained by the fact that these two antigens, Cw1 and MC1, formed a haplotype with Bw54, which suggests that one or some of the genes controlling the susceptibility and/or immune responsiveness of diffuse panbronchiolitis might be located near HLA loci. " 06/01/2004 - "Strong association between race-dependent antigens and diffuse panbronchiolitis suggests a genetic predisposition to the disease. " 02/01/1999 - "Genetic factors likely play a role in the etiology of chronic sinusitis and this disease is often associated with diffuse panbronchiolitis, which is strongly associated with HLA B54 antigen. " 03/01/1989 - "Clinical comparison of SLX with other tumor-associated antigens such as CA19-9, CA125, and CEA, was made in benign respiratory diseases including diffuse panbronchiolitis (DPB), bronchiectasis (BE), bronchial asthma (BA), and pulmonary emphysema (PE). " 03/01/1995 - "Among the bronchiolar airflow disorders, diffuse panbronchiolitis is related to HLA antigen Bw54, and low-dose, long-term erythromycin appears to be effective therapy. "
8.	Oxygen (Dioxygen)IBA 04/01/1988 - "[Nocturnal oxygen desaturation in patients with diffuse panbronchiolitis]." 09/01/1999 - "Diffuse panbronchiolitis (DPB) is a chronic inflammatory airway disease which was lethal in the past despite combined treatment with antibiotics and the use of supportive therapies such as oxygen administration. " 06/01/1998 - "Diffuse panbronchiolitis (DPB) is a chronic inflammatory disease of the airways with a high rate of mortality despite treatment with a combination of antibiotics and the use of supportive therapy such as oxygen administration. " 08/01/2023 - "Pulmonary Hypertension Associated with Diffuse Panbronchiolitis That Improved with Erythromycin and Home Oxygen Therapy."
9.	Reactive Oxygen Species (Oxygen Radicals)IBA 04/01/1997 - "EM and AZM, both of which have been shown to be clinically effective in the treatment of Diffuse panbronchiolitis (DPB), significantly suppressed active oxygen generation and chemotaxis of neutrophils at low concentrations equivalent to therapeutic doses (0.5 approximately 1.0 microgram/ml, p < 0.05), whereas the clinically ineffective RKM did not. " 01/01/2000 - "Diffuse panbronchiolitis (DPB) is characterized by chronic neutrophil-mediated inflammation of the airway mediated by oxygen radical production. " 08/01/1990 - "In order to elucidate the therapeutic mechanisms of low dose-long term erythromycin (EM) therapy in patients with diffuse panbronchiolitis (DPB), the authors evaluated the effect of in vitro EM treatment on neutrophil (PMN) oxygen radicals production. "
10.	CytokinesIBA 11/05/2004 - "The effect of long-term treatment with erythromycin on Th1 and Th2 cytokines in diffuse panbronchiolitis." 10/01/2000 - "We investigated the role of inflammatory cytokines in a mouse model of chronic Pseudomonas aeruginosa infection mimicking diffuse panbronchiolitis (DPB), and determined the effects of clarithromycin therapy on the production of these cytokines. " 02/01/1994 - "[Mechanism of efficacy of erythromycin on diffuse panbronchiolitis--effect of erythromycin on cytokine mRNA expression in human whole blood model]."

Therapies and Procedures

1.	Therapeutics 12/01/1996 - "[Pathophysiology of diffuse panbronchiolitis and progress of the therapy]." 03/10/1993 - "[Recent progress on diagnosis and therapy of diffuse panbronchiolitis]." 06/01/1992 - "EM therapy was begun in December 1983 due to a diagnosis of diffuse panbronchiolitis (DPB). " 09/01/1997 - "In conclusion, EM therapy for diffuse panbronchiolitis may be stopped if the clinical manifestations of disease (especially purulent sputum) disappear, if diffuse, nodular opacities resolve almost completely and if there is no evidence of bronchiectasis." 01/01/1997 - "Low-dose long-term erythromycin therapy has been reported to be effective in diffuse panbronchiolitis, but the mode of action remains obscure. "
2.	Lung Transplantation 10/01/2021 - "We present a case of diffuse panbronchiolitis developed after lung transplantation, which to our knowledge has not been previously described in the literature. " 10/01/2021 - "Diffuse Panbronchiolitis After Lung Transplantation: First Case in the Literature." 01/01/2016 - "We describe our experience of lung transplantation for diffuse panbronchiolitis. " 01/01/2016 - "However, lung transplantation for diffuse panbronchiolitis has rarely been reported and the outcome in these patients remains unknown. " 01/01/2016 - "Lung transplantation for diffuse panbronchiolitis: 5 cases from a single centre."
3.	Immunomodulation 01/01/2005 - "(1) Research into macrolide immunomodulation for chronic pulmonary disorders demonstrates consistent positive effects, although of types other than seen with diffuse panbronchiolitis. " 07/01/2009 - "Research into macrolide immunomodulation for chronic pulmonary disorders, such as diffuse panbronchiolitis and cystic fibrosis, shows consistent positive effects. " 01/01/2005 - "A literature search using Medline was conducted from 1966 onwards, searching for articles with relevant key words such as macrolide, diffuse panbronchiolitis, community-acquired pneumonia, biofilm, immunomodulation, cystic fibrosis, erythromycin, clarithromycin, roxithromycin and azithromycin, bronchiectasis and asthma. "
4.	Drug Therapy (Chemotherapy) 06/01/1987 - "[Clinical effects of low-dose long-term erythromycin chemotherapy on diffuse panbronchiolitis]." 12/01/2007 - "A breakthrough in antibiotic chemotherapy for patients with chronic Pseudomonas aeruginosa pulmonary infections was brought about by findings in a patient with diffuse panbronchiolitis (DPB), who had been treated with erythromycin over a period of years. "
5.	Intravenous Administration 04/01/2006 - "The treatment of refractory pneumothorax in diffuse panbronchiolitis by intravenous administration of coagulation factor XIII concentrate."